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. 2022 May 27;23(1):135.
doi: 10.1186/s12931-022-02033-6.

Comorbidity burden and survival in patients with idiopathic pulmonary fibrosis: the EMPIRE registry study

Dragana M Jovanovic  1 , Martina Šterclová  2 , Nesrin Mogulkoc  3 , Katarzyna Lewandowska  4 , Veronika Müller  5 , Marta Hájková  6 , Michael Studnicka  7 , Jasna Tekavec-Trkanjec  8 , Simona Littnerová  9 , Martina Vašáková  10 ; EMPIRE registry investigators
Collaborators, Affiliations

Comorbidity burden and survival in patients with idiopathic pulmonary fibrosis: the EMPIRE registry study

Dragana M Jovanovic et al. Respir Res. .

Abstract

Background: Patients with idiopathic pulmonary fibrosis (IPF) frequently have multiple comorbidities, which may influence survival but go under-recognised in clinical practice. We therefore report comorbidity, antifibrotic treatment use and survival of patients with IPF observed in the multi-national EMPIRE registry.

Methods: For this prospective IPF cohort, demographics, comorbidities, survival and causes of death were analysed. Comorbidities were noted by the treating physician based on the patient's past medical history or as reported during follow-up. Comorbidities were defined as prevalent when noted at enrolment, or as incident when recorded during follow-up. Survival was analysed by Kaplan-Meier estimates, log-rank test, and Cox proportional hazards models. Hazard ratios (HR) were adjusted for gender, age, smoking status and FVC at enrolment.

Results: A population of 3,580 patients with IPF from 11 Central and Eastern European countries was followed every 6 months for up to 6 years. At enrolment, 91.3% of patients reported at least one comorbidity, whereas more than one-third (37.8%) reported four or more comorbidities. Five-year survival was 53.7% in patients with no prevalent comorbidities, whereas it was 48.4%, 47.0%, 43.8% and 41.1% in patients with 1, 2, 3 and ≥ 4 comorbidities, respectively. The presence of multiple comorbidities at enrolment was associated with significantly worse survival (log-rank test P = 0.007). Adjusted HRs indicate that risk of death was increased by 44% in patients with IPF reporting ≥ 4 comorbidities at baseline compared with no comorbidity (P = 0.021). The relationship between number of comorbidities and decreased survival was also seen in patients receiving antifibrotic treatment (63% of all patients; log-rank test P < 0.001). Comorbidity as cause of death was identified in at least 26.1% of deaths.

Conclusions: The majority of patients with IPF demonstrate comorbidities, and many have comorbidity-related deaths. Increasing numbers of comorbidities are associated with worse survival; and this pattern is also present in patients receiving antifibrotic therapy.

Keywords: EMPIRE; Idiopathic pulmonary fibrosis; Mortality; Registry.

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Conflict of interest statement

The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors (ICMJE). This study was supported by Boehringer Ingelheim International GmbH (BI). BI had no role in the design, analysis or interpretation of the results in this study. BI was given the opportunity to review the manuscript for medical and scientific accuracy as it relates to BI substances, as well as intellectual property considerations. Writing/editorial support and formatting assistance was provided by Helen Keyworth, PhD, of Nucleus Global, which was contracted and funded by BI. DMJ reports personal fees from Boehringer Ingelheim and Roche outside the submitted work; and advisory board participation for Boehringer Ingelheim and Roche. NM reports consulting fees paid to their institution for advisory board participation or consultancy from Boehringer Ingelheim, Roche, Bayer and Novartis Turkey; speaker honoraria paid to their institution from Boehringer Ingelheim, Roche and Nobel; and support for congress participation from Roche, Actelion and Boehringer Ingelheim. KL reports grants, consulting fees, honoraria, support for congress participation and personal fees from Roche and Boehringer Ingelheim; and is the vice-president of the Polis IPF Patients Society. VM reports personal fees from Boehringer Ingelheim and Roche outside the submitted work; and travel grants from Boehringer Ingelheim and Roche. JTT reports personal lecture fees from Roche and Boehringer Ingelheim; and advisory board participation for Boehringer Ingelheim. MV reports personal fees or honoraria for lectures or presentations from Boehringer Ingelheim and Roche; support for conference attendance from Boehringer Ingelheim and Roche; advisory board participation for Boehringer Ingelheim; is the president of the Czech Pneumologic and Phtisiologic Society; and is the head of the EMPIRE registry. MŠ, MH, MS and SL have nothing to disclose.

Figures

Fig. 1
Fig. 1
Overall survival in patients with IPF by number of comorbidities at enrolment and during follow-up. Comorbidities (a) prevalent at enrolment and (b) cumulative at enrolment and during follow-up (both n = 3580). IPF, idiopathic pulmonary fibrosis
Fig. 2
Fig. 2
Overall survival in patients with IPF by comorbidities at enrolment according to antifibrotic therapy. Comorbidities for (a) those receiving antifibrotic therapy (n = 2257) and (b) those not receiving antifibrotic therapy (n = 1323). Antifibrotic therapies were nintedanib or pirfenidone. IPF, idiopathic pulmonary fibrosis
See this image and copyright information in PMC

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